Dilated Cardiomyopathy With Mid‐Range Ejection Fraction at Diagnosis: Characterization and Natural History

Background Limited data are available on mid‐range ejection fraction (mrEF) patients with dilated cardiomyopathy. We sought to define the characteristics, evolution, and long‐term prognosis of dilated cardiomyopathy patients with mrEF at diagnosis. Methods and Results We analyzed all dilated cardiom...

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Main Authors: Piero Gentile, Marco Merlo, Antonio Cannatà, Marco Gobbo, Jessica Artico, Davide Stolfo, Marta Gigli, Federica Ramani, Giulia Barbati, Bruno Pinamonti, Gianfranco Sinagra
Format: Article
Language:English
Published: Wiley 2019-09-01
Series:Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease
Subjects:
Online Access:https://www.ahajournals.org/doi/10.1161/JAHA.118.010705
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author Piero Gentile
Marco Merlo
Antonio Cannatà
Marco Gobbo
Jessica Artico
Davide Stolfo
Marta Gigli
Federica Ramani
Giulia Barbati
Bruno Pinamonti
Gianfranco Sinagra
author_facet Piero Gentile
Marco Merlo
Antonio Cannatà
Marco Gobbo
Jessica Artico
Davide Stolfo
Marta Gigli
Federica Ramani
Giulia Barbati
Bruno Pinamonti
Gianfranco Sinagra
author_sort Piero Gentile
collection DOAJ
description Background Limited data are available on mid‐range ejection fraction (mrEF) patients with dilated cardiomyopathy. We sought to define the characteristics, evolution, and long‐term prognosis of dilated cardiomyopathy patients with mrEF at diagnosis. Methods and Results We analyzed all dilated cardiomyopathy patients consecutively evaluated in the Trieste Heart Muscle Disease Registry from 1988 to 2013. mrEF and reduced ejection fraction (rEF) were defined as baseline left ventricular (LV) ejection fraction values between 40% and 49% and <40%, respectively. All‐cause mortality or heart transplantation, sudden cardiac death, or major ventricular arrhythmias were considered as outcome measures. Worsening LV ejection fraction (reduction to <40%) during follow‐up was also considered to identify possible predictors of adverse remodeling. Among 812 enrolled patients, 175 (22%) presented with mrEF at presentation. At baseline, as compared with the rEF group, mrEF patients had lower rates of moderate–severe mitral regurgitation and restrictive LV filling pattern. During a median follow‐up period of 120 (60–204) months, the mrEF group presented a lower rate of death/heart transplantation (9% versus 36%, P<0.001) and sudden cardiac death or major ventricular arrhythmias (4.5% versus 15%, P<0.001) than rEF patients. Moreover, 29 out of 175 mrEF patients (17%) evolved to rEF. Restrictive LV filling pattern emerged as the strongest predictor of rEF development following multivariable analysis. Conclusions mrEF identified a consistent subgroup of dilated cardiomyopathy patients diagnosed in an earlier stage with subsequent apparent better long‐term evolution. However, 17% of these patients evolved into rEF despite the use of medical therapy. A baseline restrictive LV filling pattern was independently associated with subsequent evolution to rEF.
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spelling doaj.art-6c56b73a76ce4094a6a34bed9b351f222022-12-21T18:11:42ZengWileyJournal of the American Heart Association: Cardiovascular and Cerebrovascular Disease2047-99802019-09-0181710.1161/JAHA.118.010705Dilated Cardiomyopathy With Mid‐Range Ejection Fraction at Diagnosis: Characterization and Natural HistoryPiero Gentile0Marco Merlo1Antonio Cannatà2Marco Gobbo3Jessica Artico4Davide Stolfo5Marta Gigli6Federica Ramani7Giulia Barbati8Bruno Pinamonti9Gianfranco Sinagra10Cardiothoracic Department Azienda Sanitaria Universitaria Integrata di Trieste and University of Trieste ItalyCardiothoracic Department Azienda Sanitaria Universitaria Integrata di Trieste and University of Trieste ItalyCardiothoracic Department Azienda Sanitaria Universitaria Integrata di Trieste and University of Trieste ItalyCardiothoracic Department Azienda Sanitaria Universitaria Integrata di Trieste and University of Trieste ItalyCardiothoracic Department Azienda Sanitaria Universitaria Integrata di Trieste and University of Trieste ItalyCardiothoracic Department Azienda Sanitaria Universitaria Integrata di Trieste and University of Trieste ItalyCardiothoracic Department Azienda Sanitaria Universitaria Integrata di Trieste and University of Trieste ItalyCardiothoracic Department Azienda Sanitaria Universitaria Integrata di Trieste and University of Trieste ItalyBiostatistics Unit Department of Medical Sciences University of Trieste ItalyCardiothoracic Department Azienda Sanitaria Universitaria Integrata di Trieste and University of Trieste ItalyCardiothoracic Department Azienda Sanitaria Universitaria Integrata di Trieste and University of Trieste ItalyBackground Limited data are available on mid‐range ejection fraction (mrEF) patients with dilated cardiomyopathy. We sought to define the characteristics, evolution, and long‐term prognosis of dilated cardiomyopathy patients with mrEF at diagnosis. Methods and Results We analyzed all dilated cardiomyopathy patients consecutively evaluated in the Trieste Heart Muscle Disease Registry from 1988 to 2013. mrEF and reduced ejection fraction (rEF) were defined as baseline left ventricular (LV) ejection fraction values between 40% and 49% and <40%, respectively. All‐cause mortality or heart transplantation, sudden cardiac death, or major ventricular arrhythmias were considered as outcome measures. Worsening LV ejection fraction (reduction to <40%) during follow‐up was also considered to identify possible predictors of adverse remodeling. Among 812 enrolled patients, 175 (22%) presented with mrEF at presentation. At baseline, as compared with the rEF group, mrEF patients had lower rates of moderate–severe mitral regurgitation and restrictive LV filling pattern. During a median follow‐up period of 120 (60–204) months, the mrEF group presented a lower rate of death/heart transplantation (9% versus 36%, P<0.001) and sudden cardiac death or major ventricular arrhythmias (4.5% versus 15%, P<0.001) than rEF patients. Moreover, 29 out of 175 mrEF patients (17%) evolved to rEF. Restrictive LV filling pattern emerged as the strongest predictor of rEF development following multivariable analysis. Conclusions mrEF identified a consistent subgroup of dilated cardiomyopathy patients diagnosed in an earlier stage with subsequent apparent better long‐term evolution. However, 17% of these patients evolved into rEF despite the use of medical therapy. A baseline restrictive LV filling pattern was independently associated with subsequent evolution to rEF.https://www.ahajournals.org/doi/10.1161/JAHA.118.010705dilated cardiomyopathyheart failuremid‐range ejection fractionprognosis
spellingShingle Piero Gentile
Marco Merlo
Antonio Cannatà
Marco Gobbo
Jessica Artico
Davide Stolfo
Marta Gigli
Federica Ramani
Giulia Barbati
Bruno Pinamonti
Gianfranco Sinagra
Dilated Cardiomyopathy With Mid‐Range Ejection Fraction at Diagnosis: Characterization and Natural History
Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease
dilated cardiomyopathy
heart failure
mid‐range ejection fraction
prognosis
title Dilated Cardiomyopathy With Mid‐Range Ejection Fraction at Diagnosis: Characterization and Natural History
title_full Dilated Cardiomyopathy With Mid‐Range Ejection Fraction at Diagnosis: Characterization and Natural History
title_fullStr Dilated Cardiomyopathy With Mid‐Range Ejection Fraction at Diagnosis: Characterization and Natural History
title_full_unstemmed Dilated Cardiomyopathy With Mid‐Range Ejection Fraction at Diagnosis: Characterization and Natural History
title_short Dilated Cardiomyopathy With Mid‐Range Ejection Fraction at Diagnosis: Characterization and Natural History
title_sort dilated cardiomyopathy with mid range ejection fraction at diagnosis characterization and natural history
topic dilated cardiomyopathy
heart failure
mid‐range ejection fraction
prognosis
url https://www.ahajournals.org/doi/10.1161/JAHA.118.010705
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